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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 657

Retiform hemangioendothelioma over forehead: A rare tumor treated with chemoradiation and a review of literature

1 Department of Surgical Oncology, Tata Memorial Centre, Mumbai, Maharashtra, India
2 Department of Head and Neck Surgical Oncology, Tata Memorial Centre, Mumbai, Maharashtra, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Prathamesh Pai
Department of Head and Neck Surgical Oncology, Tata Memorial Centre, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.148693

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 > Abstract 

Retiform hemangioendothelioma (RH) is low grade tumor of skin and subcutaneous tissue. It needs to be differentiated from angiosarcoma as RH has excellent prognosis. It is usually seen in young adults on extremities. Sometimes it may mimic benign conditions and can delay treatment. Surgery has been mainstay of its treatment with or without adjuvant radiation. We present first case of RH on face. This is only second case being treated with definitive chemoradiation. So it's important to distinguish RH from angiosarcoma due to treatment implications as well.

Keywords: Chemoradiation, face, retiform hemangioendothelioma

How to cite this article:
Tamhankar AS, Vaidya A, Pai P. Retiform hemangioendothelioma over forehead: A rare tumor treated with chemoradiation and a review of literature. J Can Res Ther 2015;11:657

How to cite this URL:
Tamhankar AS, Vaidya A, Pai P. Retiform hemangioendothelioma over forehead: A rare tumor treated with chemoradiation and a review of literature. J Can Res Ther [serial online] 2015 [cited 2021 Apr 15];11:657. Available from: https://www.cancerjournal.net/text.asp?2015/11/3/657/148693

 > Introduction Top

Retiform hemangioendothelioma (RH) was first described in 1994, differentiating it from angiosarcoma. It is usually seen in young adults on the extremities. It manifests as a cutaneous plaque or subcutaneous nodule that is either asymptomatic or causes local discomfort. Typically, the histology shows proliferation of arborizing blood vessels. RH differs from angiosarcoma in lacking cytologic atypia and high mitotic rates. This neoplasm grows slowly and frequently recurs, but rarely metastasizes. This is the 30 th case of RH being reported in the literature and the first one on the face. It is only the 2 nd case in the literature to have been treated by definitive chemoradiation.

 > Case report Top

A 48-year-old housewife presented with irregular plaque-like thickening of skin over left forehead since 18 months. Diagnosis of leprosy was made at a community medical center due to appearance of the lesion and she was treated with multidrug therapy consisting of Dapsone and Rifampicin. The lesion failed to respond and continued to progress to involve left temple, forehead, and the cheek [Figure 1]. There was no pain in the lesion. There was no history of paresthesias and neurodeficit or similar lesions on other parts of the body. On examination, the lesion was irregular, measuring about 11 × 7 cm in size, and had an erythematous surface with peau d'orange appearance. It involved the left supra-orbital region, temple, eyelid, infra-orbital region, and zygoma. It was firm and non-compressible. There was another lesion in the retroauricular region. There was no cervical lymphadenopathy. Magnetic resonance imaging (MRI) scan revealed irregular skin and subcutaneous mass over temporal fossa, free from underlying bone. Metastatic work-up was normal. Skin biopsy of the temporal lesion revealed retiform vascular proliferation within the dermis and superficial subcutaneous tissue [Figure 2]. The endothelial cells of these vessels had hobnail appearance. These cells expressed CD31 and D240, while the immunostains CD34, AE1/AE3, and HHV8 were negative [Figure 3]. This appearance was suggestive of RH. Retroaural lesion was low-grade angiosarcoma.
Figure 1: Pre-treatment appearance of the lesion over the temple (All citations of figures mentioned in text)

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Figure 2: H and E stain showing arborizing blood vessels (retiform pattern)

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Figure 3: CD-31 positive tumor cells

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The lesion had extensive skin and subcutaneous involvement, and surgical resection of the lesion would have amounted to significant disfigurement which would have been unacceptable. The case was discussed in tumor board (Disease Management Group), and patient was planned for concurrent chemoradiation with Cisplatin (30 mg/m 2 weekly) and radiotherapy of 50 Gy in 25 fractions. Retroaural lesion was excised with negative margins. The field of radiation did not include retroauricular area as it was resected with negative margins. The lesion regressed significantly in dimensions and thickness. Patient is progression free at the end of 3 months of follow-up with good cosmetic outcome [Figure 4].
Figure 4: Post-treatment regression of the lesion with good cosmetic outcome

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 > Discussion Top

RH is a low-grade tumor of vascular origin resembling cutaneous angiosarcoma (CA). Calonje et al. [1] differentiated it from CA in 1994. Hirsh et al. presented a review in 2010 of 25 similar cases. [2] Since then, four more cases have been reported in English literature. [3],[4],[5],[6] Two cases with lesions on scalp have been reported until now. [1],[3] This is the first case being reported on the face. Only one patient has been treated definitively with primary chemoradiation before this.

This unusual tumor occurs mainly in the lower limbs. Few cases have been reported with tumors in the trunk, head, and penis. [1],[7] It is most common in young or middle-aged adults, with a greater frequency in females. [1],[7] Distant metastases are rare. But local recurrences are seen in about 50% cases. [7] Tumor-related death has been reported in only one patient. [3]

RH mostly presents as an asymptomatic, slow-growing single lesion. [3] However, multiple, rapid-growing lesions have been reported. [3],[4],[8] Lesion is usually indolent. It grows over a variable period of time ranging from few months to several years. [7] Most cases present as exophytic, dermal, or subcutaneous nodules or plaques with a size range of 1-30 cm. [7]

The etiology of this rare entity remains unknown. However, several reports have proposed its association with human herpesvirus-8, prior lymphangioma lymphedema, previous radiation treatment, and nonepidermal malignant tumors. [1],[2]

As there is no diagnostic feature characteristic for this tumor, biopsy is the only diagnostic method available at present. [1],[7],[9] Histologically, it is characterized by long arborizing vascular channels lined with hobnail endothelial cells. The vascular channels are arranged in a retiform pattern resembling rete testis, hence the name. Prominent stromal lymphocytic infiltrates are often observed. On immunohistochemistry, these cells express endothelial markers such as CD31, CD34, factor VIII, and Ulex europaeus agglutinin-1. [1] The differential diagnosis of RH includes Dabska's tumor (dabska's tumor is a separate entity. Don't change it everywhere.), CA, malignant lymphoma, dermatofibrosarcoma protuberans, bacillary angiomatosis, Kaposi's sarcoma, and targetoid hemosiderotic hemangioma. [1],[2],[7] CA is a highly aggressive neoplasm with a very high incidence of local recurrence and metastasis and a high mortality rate. [10],[11] Angiosarcoma differs from RH in having significant cytologic atypia, presence of conspicuous mitotic figures, tumor infiltrating between individual collagen bundles, and absence of hobnail endothelial cells. [10],[11] RH and Dabska's tumor are biologically similar, and some authors have even termed RH as the adult counterpart of Dabska's tumor and have included both of them under the heading of "hobnail hemangioendothelioma [7]". Unlike RH, Dabska's tumor occurs mostly in children and has slight predilection for the head and neck area. Histologically, Dabska's tumor has well-formed papillary endothelial projections and lacks arborizing rete testis-like architecture. Cutaneous angiomatosis usually occurs in infants or young adults, and often involves a large segment of the body. Histologically, angiomatosis consists of large venous, cavernous, as well as capillary-sized vessels that are usually located in the wall or adjacent to a large vein. [12]

Treatment of RH is controversial. Wide surgical excision with histopathologically free margins is performed whenever feasible. Long-term follow-up is mandatory owing to the high propensity of recurrences. Radiation therapy has been reported to be successful in two cases with lymph node metastasis. [1] The earlier case reported from India had a lesion over mons pubis with lymph node metastases. The lesion was resected with lymph node dissection, and postoperative radiotherapy was given. Patient defaulted after recurrence. [9] One case of unresectable RH has been successfully treated with low-dose Cisplatin and moderate radiotherapy. Patient remained disease free at 36 months. [2] Interferon alpha has also been tried as immunotherapy. So, surgery, radiotherapy, and both combined have produced mixed results. The number of cases available in the literature is too small to set up guidelines for treating this entity at this point of time. The follow-up of the patient is relatively short to comment on the definite outcome variables. This entails further study of similar cases for deciding the most optimal treatment modality.

 > Conclusion Top

RH is a rare low-grade malignant tumor. It is important to distinguish it from its aggressive counterpart, i.e. angiosarcoma, and they differ (new sentence is allright.) markedly in their behavior and prognosis. Physician needs to be aware of its potential of mimicking benign conditions like leprosy, as in this report. With accumulation of evidence, chemoradiation may emerge as the treatment of choice in this indolent malignancy. Surgery may play a major role in smaller, resectable lesions, whereas radiotherapy might be reserved for future recurrences in areas that are cosmetically less important. This is the first case of RH being reported on face. Further, this is only the 2 nd case to be treated by primary chemoradiation. Further reporting of similar cases and their treatment modalities might give us a better insight into the disease biology and treatment responsiveness.

 > References Top

Calonje E, Fletcher CD, Wilson-Jones E, et al. Retiform hemangioendothelioma: A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115-25.  Back to cited text no. 1
Hirsh AZ, Yan W, Wei L, et al. Unresectable retiform hemangioendothelioma treated with external beam radiation therapy and chemotherapy: A case report and review of the literature. Sarcoma 2010;2010:756246. http://dx.doi.org/10.1155/2010/756246.  Back to cited text no. 2
Zhang, et al. A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features. Int J Clin Exp Pathol 2010;3:528-33.  Back to cited text no. 3
WK Choi, SH Lee, SA Oh, DH Kang. Retiform Hemangioendothelioma on the Finger. Archives of Plastic Surgery. 39; Jan 2012 81.  Back to cited text no. 4
Albertini, Anne-Fore MD, et al. American J of Dermatopathol 33:e84-e87, Oct 2011.  Back to cited text no. 5
Retiform hemangioendothelioma treated with Mohs micrographic surgery Susan A. Keiler, MD et al. J of the American Academy of Dermatology 2011;65:233-5.  Back to cited text no. 6
Tan D, Kraybill W, Cheney RT, et al. Retiform hemangioendothelioma: A case report and review of the literature. J Cutan Pathol 2005;32:634-7.  Back to cited text no. 7
Duke D, Dvorak A, Harris TJ and Cohen LM. Multiple retiform hemangioendotheliomas. A lowgrade angiosarcoma. Am J Dermatopathol 1996;18:606-10.  Back to cited text no. 8
Bhutoria B, Konar A, Chakrabarti S, et al. Retiform hemangioendothelioma with lymph node metastasis: A rare entity. Indian J Dermatol Venereol Leprol 2009;75:60-2.  Back to cited text no. 9
[PUBMED]  Medknow Journal  
Cooper PH. Angiosarcomas of the skin. Semin Diagn Pathol 1987; 4:2-17.  Back to cited text no. 10
Alessi E, Sala F and Berti E. Angiosarcomas in lymphedematous limbs. Am J Dermatopathol 1986;8:371-8.  Back to cited text no. 11
Rao VK and Weiss SW. Angiomatosis of soft tissue. An analysis of the histologic features and clinical outcome in 51 cases. Am J Surg Pathol 1992; 16:764-71.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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